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[Improved care for the aphasic patient: introduction to the central theme of aphasia].

The Dutch Hart Foundation has as one of its major goals the furthering of revalidation of hart patients as well as patients with cerebrovascular illnesses. Since many of these latter patients suffer from aphasic disturbances, the CVD-committee of the above mentioned foundation has, in co-operation with the Dutch Society for Neurology, investigated the possibilities of improving the treatment of these disturbances. A systematic improvement of this treatment depends on the ability to measure it. Therefore, the foundation has invited a number of prominent researchers in this area for a workshop in August 1979 posing the question whether and if so how the severity of aphasia can be measured. The lectures of this meeting, including the discussion, are published in this issue.

Aged↗

[A syndrome of visuo-verbal and visuo-gestural disconnexion. Optic aphasia and optic apraxia (author's transl)].

A 71 year-old female patient was investigated during several months after a double left hemispheric lesion of vascular origin. The larger lesion was temporo-occipital, the second one parietal. The main neurological disorder was a right homonymous hemianopia. Clinical and neuroradiological data confirmed the integrity of the right hemisphere. There was neither a global deterioration of intellectual capacities nor aphasia. In particular, visual agnosia was excluded by a series of matching and functional classification tests. Nevertheless the objects and their pictures, which the patient could recognize, could not be named. The lack of words was limited to the visual modality. No apraxia neither ideomotor nor ideational was present on verbal commands, but the evocation of movements for the use of objects was deficient if the stimulation was visual. This disorder is considered in the frame of the visual-gestural disconnection and thus represents what we propose to call visual apraxia. Further we discuss the disorders of constructive apraxia which are characterized by the poor reproduction of models when executed by the right hand whereas the reproduction by the left hand is incorrect.

Aged↗

Landau-Kleffner syndrome: acquired epileptic aphasia in children.

Acquired epileptic aphasia, or Landau-Kleffner syndrome (LKS), once thought to be a rare syndrome, may occur more frequently in the pediatric population than once thought. This syndrome is typically characterized by an abrupt or gradual loss of language ability and inattentiveness to sound, sometimes called auditory agnosia, with onset during the first 5 years of life. This interruption in communication ability is generally closely preceded, accompanied, or followed by the onset of seizure activity and/or abnormal electro-encephalographic (EEG) findings. This report describes two cases of LKS evaluated at the same hearing and speech center. Because of the characteristic language regression and inattentiveness to sound, speech-language pathologists and audiologists are likely to be among the first professionals to evaluate these children. It is imperative that communication specialists be alert to the characteristic symptoms of LKS in order to be instrumental in initiating an appropriate diagnostic and management process. A multidisciplinary approach to identification and rehabilitation is encouraged in order to effectively re-establish communication skills for these children.

Aphasia↗

[The MASA syndrome (Mental retardation, Aphasia, Spastic paraplegia and Adducted thumbs), is it heterogeneous?].

BACKGROUND: MASA syndrome is the acronym for Mental retardation, Aphasia, Shuffling gait and Adducted thumbs. Linkage studies have shown linkage to markers in the Xq28 band. CASE REPORTS: Case no. 1: Mickaël was examined at the age of 3 yr 4 mo. He was mentally retarded (IQ = 40), aphasic, and had spastic gait, moderate facial dysmorphy and adducted thumbs. His parents were normal, except that his mother had similar facial dysmorphy. His brain CT scan was normal. Case no. 2: Philippe was the elder brother of Mickaël. When examined at the age of 5 years, he had the same features as his brother. His IQ was 40. His brain-CT scan was also normal. DNA analysis with markers for the Xq28 area showed that the brothers had received different X chromosomes from their mother. CONCLUSION: DNA studies suggest that the MASA syndrome is heterogeneous.

Aphasia↗

[The seizure syndrome combined with sensorimotor aphasia and alalia (the Landau-Kleffner syndrome)].

Electric brain activity and clinical status were studied in children with Landau-Kleffner syndrome. The paper covers 21 cases of the disease in patients aged from 2 years 11 months to 16 years in whom speech disorders and convulsive episodes varied in onset. A characteristic EEG pattern was recorded in the majority of the examinees. It comprised slow high-amplitude spike-and-wave complexes localized primarily in the temporal regions, more frequently on the left side. Clinically, the children belonged to 2 groups: epileptic aphasia and epileptic alalia patients. The latter had more significant EEG changes attributed to earlier onset of this disease. The data obtained showed the importance of combined clinical and EEG investigations in the diagnosis of Landau-Kleffner syndrome.

Adolescent↗

[Dissociated preservation of written expression in aphasia with recurrent utterances].

In a female patient, aphasia with recurrent utterances resulted from a double lesion of the left hemisphere, located in the base of the third frontal gyrus and the upper temporal gyri. Predominant in the fluent oral expression was a repeated neologism of which the patient was unaware. The written expression, efficient for lexical entities, was devoid of syntax and had the features of phonological agraphia. Both orally and in written words comprehension was satisfactory but syntactic comprehension was deficient. The stereotyped verbal behaviour could be due to dysfunction of the phonemic programmer. Rupture of the audi-phonatory loop, confirmed by degradation of the verbal working, memory, made this dysfunction worse and accounted for the anosognosia. The participation of the right hemisphere and of the preserved left hemispheric structures in the persistence of various language activities are discussed.

Aphasia↗

Aphasia in Maurice Ravel.

A selective loss of language resulting from left hemisphere cerebral lesions is familiar to all neurologists but only rarely does such a deficit allow preexisting extraordinary capabilities of the right hemisphere to emerge. A retrospective case history of French composer Maurice Ravel demonstrates such a right-sided cognitive system. At 58, Ravel was struck with aphasia, which quelled any further artistic output. Most strikingly, he was able to think musically but unable to express his ideas in either writing or performance. Hemispheric lateralization for verbal (linguistic) and musical thinking offers an explanation for the dissociation of Ravel's ability to conceive and to create. What makes Ravel's history interesting to the public as well as to physicians is not only the tragic toll exacted in this composer's personal and creative life but also the resultant loss of the output of one of the 20th century's towering musical geniuses.

Aphasia↗

Recovery from aphasia and conversion of handedness: a case report.

A remarkably rapid and complete recovery from aphasia and conversion from right to left handedness was documented in a hemiplegic individual after surgery for an arteriovenous malformation (AVM) in the dominant hemisphere. The possible mechanisms of recovery were discussed and we have reviewed the literature about the pattern of cerebral dominance. Her rapid recovery could be explained by the co-dominance of the right and left cerebral hemispheres.

Adult↗

Acute aphasia complicating typhoid fever in an adult.

Motor aphasia complicating bacteriologically confirmed typhoid fever in a 20-year-old adult female is presented. Neither the cause of death nor the aetiology of this isolated neurological deficit could be determined from detailed post-mortem examination. An immune related mechanism is suggested based on the temporal characteristics of this complication and CSF abnormalities.

Acute Disease↗

[Anterior choroidal artery territory infarction with transcortical sensory aphasia after a neck clipping of left internal carotid artery aneurysms: a case report].

Anterior choroidal artery (Ach A) syndrome is widely known to be composed of a triad of hemiparesis, hemisensory loss and homonymous hemianopia. The authors report a case of Ach A-territory infarction after a direct operation for internal carotid artery aneurysms, which presented transcortical sensory aphasia in association with the characteristic triad. The literature and several previously reported cases with dominant and nondominant cerebral hemisphere signs in AcH A infarction are reviewed. The danger of Ach A occlusion and the importance of preserving it during aneurysm surgery are emphasized.

Aneurysm↗

Language therapy effects in long term aphasia.

This report describes the results of language therapy initiated 1 to 6 years after the onset of aphasia in 14 patients. During the course of treatment, each of the 14 patients improved strongly in their communicative abilities (PICA), according to clinical observation and reports from family, hospital ward personnel, or both.

Adult↗

[An unusual autopsy case of corticobasal degeneration--with special reference to clinicopathological differentiation from progressive supranuclear palsy and slowly progressive aphasia].

We report an unusual autopsy case of corticobasal degeneration. The patient was male who was 67 years old at the time of his death. He developed clumsiness of his right hand at age 65. Neurological examination at age 66 revealed supranuclear ophthalmoplegia of upward gaze and parkinsonism. Progressive supranuclear palsy was suspected. The patient's clinical course was rapidly progressive. Dementia developed nine months after the onset of the disease, and the patient manifested apallic syndrome at 21 months and died of pneumonia at about 25 months. At autopsy the brain weighed 1370g. Macroscopic examination revealed prominent atrophy of the anterior and parietal lobes, particularly of the pars opercularis in the left inferior frontal gyrus, and there was marked depigmentation of the substantia nigra. Histological examination showed neuronal loss, glial proliferation, spongy state, and ballooned neurons in the frontal and parietal lobes. Neuronal loss with glial proliferation was conspicuous in the striatum, pallidum, thalamus, and substantia nigra. There was slight neuronal loss in the dentate nucleus and locus ceruleus. Staining by Gallyas-Braak method revealed massive appearance of argyrophilic threads. This case is important in terms of the clinical differentiation from progressive supranuclear palsy, and is also interesting in regard to the pathological correlation with slowly progressive aphasia.

Aged↗

Primary progressive aphasia.

Primary progressive aphasia is an important recently emphasized clinical syndrome that is a common early manifestation of Pick's disease and Pick complex pathology. It is defined clinically as slowly progressive language disturbance that remains relatively isolated from other cognitive or behavioral deficits for at least 2 years. Subsequently, it may become associated with behavioral changes similar to those in frontal lobe dementia (FLD), extrapyramidal manifestations, and apraxia similar to corticobasal degeneration (CBD) and with motor neuron disease. Both the common nonfluent or logopenic variety leading to mutism (frontal involvement) and fluent or semantic varieties (temporal involvement) are recognized. Neuroimaging with CT, MRI, and SPECT are useful adjuncts confirming the diagnosis. PPA is a manifestation of frontotemporal degeneration or Pick complex, which is probably the second most common degenerative dementia after AD.

Aphasia, Primary Progressive↗

Computer rehabilitation in aphasia.

Recent work in applying computers to treatment of aphasia demonstrates some promising early results, but marketing of therapy programs is far ahead of efficacy studies. The need for rigorous controlled studies and development of therapy programs based on theoretically sound principles and neuropsychological models remains acute.

Aphasia↗

[Aphasia in a polyglot: description and neuropsychological course].

We present a case of aphasia due to an ischaemic lesion in the left temporo-occipital region of the brain of a 60 year old right-handed polyglot. Mother tongues: French, Italian, Arabic. Educated at school in English. Languages learnt as an adult: German, Portuguese, Spanish. Language habitually spoken prior to illness: Spanish. His language disorder was of non-fluent type and progressed to an anomic disorder. The non-parallel recovery of languages led to an initial and predominant recovery of English (language at school) followed by French (his first language). This type of non-parallel recovery may be compatible with the inhibition-disinhibition mechanism hypothesis. This would mean that the languages of least recovery are inhibited by raising the threshold of some circuits while still permitting comprehension.

Aphasia↗

[Mixed transcortical aphasia due to multiple deep white matter infarction in the dominant cerebral hemisphere: a case report].

We report a case of mixed transcortical aphasia (MTA) due to multiple cerebral infarction in the dominant hemisphere in an 80-year-old right-handed woman without hemiplegia. Her spontaneous speech was markedly reduced and auditory comprehension, reading and writing were severely disturbed. Although the repetition of sentences (at most 3 words) was relatively preserved, her speech was echolalic. Brain MRI showed bilateral multiple deep white matter infarction and subcortical infarction of the left parietal lobe, including left angular gyrus, but no abnormal signal intensities were detected in either Wernicke's or Broca's area. SPECT indicated a significant decrease in mean cerebral blood flow in both hemispheres, but there was no focal hypoperfusion in either speech area. We thought that the focal hypoperfusion observed in the right cerebellum indicated crossed cerebellar diaschisis. Electroencephalogram showed a diffuse reduction in the incidence of alpha waves in the left cerebral hemisphere. From these findings, we suggest that widespread hypofunction in the dominant hemisphere was important for the occurrence of MTA.

Aged↗

Semantic field in aphasia: an experimental investigation on comprehension of the relations of class and property.

A multiple choice test was devised in order to assess some aspects of the aphasic's semantic field; patient's task was to match a given picture with one out of three alternatives: one of them was linked by a class relationship, one was linked by a property relationship and the last was an unrelated item. Results clearly show that Broca's aphasics make their choice according to class criteria whereas Wernicke's and controls make their choice according to property criteria. Results are discussed in the light of Jakobson linguistic hypothesis which postulates that Broca's and Wernicke's aphasia are subdued by a contiguity and similarity disorder.

Aphasia↗